[Serum IgG in some collagen diseases and in pemphigus].
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Four cases of fatal strongyloidiasis were reported from Thailand; two were malnourished young inmates from the same Government-Institution while two were adults receiving corticosteroid therapy for pemphigus vulgaris and systemic lupus erythematosus. The clinical and pathological findings of all cases were briefly described. Special emphasis is made in treatment of malnourished young children especially those living in the institution with possibility of such parasitic hyperinfection superimposed by other organisms. A single course of thiabendazole in strongyloidiasis is inadequate, especially in those immunosuppressive hosts.
Fifty patients with various kinds of skin diseases who were not adequately relieved by conventional therapy were treated with ACTH or cortisone given systemically. Almost all patients with disseminated neurodermatitis had dramatic initial response, but in only about half the cases was improvement maintained when use of the drugs was discontinued. It appeared that in other skin diseases, such as lupus erythematosus, scleroderma, psoriasis, dermatomyositis and pemphigus, while improvement may be noted for a time, relapse to the original state occurs after the treatment is stopped. In four cases of chronic discoid lupus erythematosus, although some improvement was observed when steroid therapy was given, the histologic pattern of biopsy material taken from the lesions after treatment still was characteristic of the disease.
This is a retrospective analysis of patients with pemphigus vulgaris and connective tissue disease (CTD) present as systemic lupus erythematosus, mixed CTD, or both. Pemphigus vulgaris was severe, difficult to treat, but eventually responded to therapy, resulting in a remission. In 6 patients, the CTD was stable and controlled with symptomatic therapy, and in 7 patients required systemic therapy. Life-threatening systemic involvement in systemic lupus erythematosus or mixed CTD-such as renal, cardiac, and neurologic-were absent in these patients on 8 years (range 3-18 years) of follow-up. There are two limitations to this study. First, it is a retrospective study. Second, no other disease control groups were used for comparison purposes.
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Immunofluorescent tests have proved to be of diagnostic importance for pemphigus, bullous pemphigoid, cicatricial pemphigoid, systemic lupus erythematosus, and discoid lupus erythematosus. Immunofluorescence test procedures, necessary specimens, and test findings have been reviewed as aids to dentists in the utilization and interpretation of these tests for the study of oral lesions.
The indirect immunofluorescence test has acquired great importance in the diagnosis of auto-immune skin diseases. Since it is sufficient only to send a sample of whole blood or blood serum to carry out the test, it is also of practical interest to the general practitioner. The test should be called upon if any of the following auto-immune skin diseases are suspected: pemphigus vulgaris (including p. vegetans, p. foliaceus, p. erythematosus), bullous pemphigoid (including benign mucosal pemphigoid) lupus erythematodes (systemic) and diffuse scleroderma.
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A 35-year-old man presented with a generalized bullous eruption and oral ulceration. He had been diagnosed as having systemic lupus erythematosus and pelvic Castleman disease (hyaline-vascular type) in the past. Histologic, direct and indirect immunofluorescence and immunoprecipitation studies confirmed a diagnosis of paraneoplastic pemphigus (PNP). Initially several medical treatments were tried unsuccessfully. The pelvic tumor was surgically removed and the mucocutaneous lesions slowly regressed. Four years after presentation, he developed polymyositis which was completely controlled with short courses of corticosteroids. There was no evidence of relapse of PNP or lupus erythematosus at that time. At the 6-year follow-up he showed no clinical evidence of PNP, lupus erythematosus or polymyositis without requiring immunosuppressive therapy. This case emphasizes the fact that patients with benign-neoplasm-associated PNP may undergo complete remission of the autoimmune disease upon complete excision of the tumor. This case also stresses the possible association of PNP with other autoimmune diseases such as lupus erythematosus and polymyositis.
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The capability of complement fixation of pemphigus antibodies was tested using combined in vitro complement immunofluorescent (IF) staining methods. Three sera out of 25 serum samples from 22 pemphigus patients revealed positive reactions, while all other sera gave negative results. Specificity control tests confirmed the positive reactions to be specific for complement staining. Complement fixing pemphigus antibodies were titrated lower than corresponding IgG antibodies and were demonstrable only in the extensive stage of the disease. Thus, the present work supplied evidence that pemphigus antibodies fix complement in vitro. However, the discrepancy still remains between the in vivo deposition of complement in most cases of pemphigus and in vitro capability of complement fixation in only few cases. More investigations should be needed to explain the exact role of complement in pemphigus acantholysis.
A 49-year-old woman with systemic lupus erythematosus, and a history of thymoma and chronic white plaques of the oral mucosa, developed a blistering eruption which was diagnosed as pemphigus foliaceus based on clinical, histological and direct immunofluorescence findings. However, the white plaques had both the clinical and histological features of lichen planus, as well as intercellular antibodies suggestive of pemphigus. Such conditions in combination have been documented previously, but this is believed to be the first report of the coexistence of all four in one individual. The common mechanism underlying these four conditions may well be an immunological disturbance.
Sera of 532 patients with bullous diseases, connective tissue diseases and malignancies were tested for pemphigus epidermal intercellular fluorescence (ICF) and for the bullous pemphigoid ;tubular' band by the indirect fluorescent antibody technique. Human normal skin cryostat sections were used. The band and ICF were seen primarily only in bullous pemphigoid and pemphigus respectively, Some indirect band and ICF-negative patients demonstrated positive direct results in involved skin. suggesting that direct tests should be performed in indirect negative patients clinically thought to have pemphigus or bullous pemphigoid. No close correlation was found between disease activity and positive or negative indirect tests in bullous pemphigoid and pemphigus. Steroids did not interfere with positive results of this diagnostically extremely valuable test.
The clinical findings of pemphigus vulgaris, rheumatoid arthritis, and Sjögren's syndrome were found in a patient displaying immunological abnormalities found in pemphigus vulgaris, systemic lupus erythematosus, rheumatoid arthritis, and Hashimoto's thyroiditis. Some of the antigen-antibody systems detected in this case were further identified. Our patient may be an example of severe failure in immune regulation.
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A case of a woman of 23 years old with lesions in the exposed areas of the skin (face and lower neck) is presented. The histological study was compatible with pemphigus (subcorneal acantholysis and acantholytic cells). The direct immunofluorescence revealed the presence of IgG in intercellular spaces on all the surfaces of the epidermis. In the indirect immunofluorescence ANA (1/800), anti-DNA (1/200) and anti-ICS (1/200) was found. The rest of the analysis clearly showed thrombocytopenia and C4 consumption. The interesting aspect of this case rests in the coexistence of clinical and analytical findings of each of these of autoimmune diseases.
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